Idiopathic multicentric osteolysis with nephropathy
Journal
Journal of the Formosan Medical Association
Journal Volume
99
Journal Issue
3
Pages
243-247
Date Issued
2000
Author(s)
Abstract
Idiopathic multicentric osteolysis is a rare syndrome that manifests with progressive loss of carpal and tarsal bones in childhood. Affected children have arthritic-like episodes, followed by progressive deformities, radiographic osteolytic changes, and variable degrees of disability. A rare form of this disease (type III, sporadic) is associated with serious nephropathy. We present the first reported case of type III idiopathic multicentric osteolysis in a Chinese woman. The patient, a 34-year-old woman with normal mental development and no family history of bone or kidney disease, presented with a 4-day history of nausea and vomiting. She had shortening anti swelling of the hands, which had occurred in childhood and persisted at the time of admission. X-ray studies showed disappearance of the carpal bones, and multiple osseous erosions of the tarsal bones. Hypertension, severe azotemia, and metabolic acidosis were also noted. Advanced renal disease was documented after a series of investigations, including renal biopsy. She is now dialysis-dependent. This case illustrates the importance of early diagnosis and management of idiopathic multicentric osteolysis with nephropathy.
Subjects
Glomerulosclerosis; Idiopathic multicentric osteolysis; Nephropathy
SDGs
Other Subjects
adult; article; bone malformation; carpal bone; case report; childhood disease; clinical feature; dialysis; disease classification; early diagnosis; female; human; idiopathic disease; kidney disease; osteolysis; tarsal bone; Adult; Female; Humans; Kidney Diseases; Osteolysis, Essential
Type
journal article